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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
32
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pubmed:dateCreated |
1991-1-8
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pubmed:abstractText |
Human beta-mannosidosis urine was fractionated by gel permeation chromatography on Bio-Gel P-2 and by high performance liquid chromatography on Partisil 10 SAX. Besides the disaccharide Man beta 1-4GlcNAc as the major component, a sialic acid-containing compound was detected in an amount of 10% compared to that of Man beta 1-4GlcNAc. Structural characterization of the oligosaccharide and of its reduced analogue by sugar composition analysis, methylation analysis, gas-liquid chromatography-mass spectrometry, and 500-MHz 1H NMR spectroscopy gave conclusive evidence for a novel urinary constituent: NeuAc alpha 2-6Man beta 1-4GlcNAc. This linear trisaccharide can be considered as the result of an alpha 2-6-sialylation of the major accumulating compound, Man beta 1-4GlcNAc. The hitherto unknown linkage between sialic acid and mannose was shown to be susceptible to sialidase digestion.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Nov
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pubmed:issn |
0021-9258
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pubmed:author |
|
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pubmed:issnType |
Print
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pubmed:day |
15
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pubmed:volume |
265
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
19685-9
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:2246252-Carbohydrate Sequence,
pubmed-meshheading:2246252-Carbohydrates,
pubmed-meshheading:2246252-Chromatography, Gel,
pubmed-meshheading:2246252-Chromatography, High Pressure Liquid,
pubmed-meshheading:2246252-Chromatography, Thin Layer,
pubmed-meshheading:2246252-Gas Chromatography-Mass Spectrometry,
pubmed-meshheading:2246252-Humans,
pubmed-meshheading:2246252-Magnetic Resonance Spectroscopy,
pubmed-meshheading:2246252-Male,
pubmed-meshheading:2246252-Mannose,
pubmed-meshheading:2246252-Mannosidases,
pubmed-meshheading:2246252-Methylation,
pubmed-meshheading:2246252-Molecular Sequence Data,
pubmed-meshheading:2246252-N-Acetylneuraminic Acid,
pubmed-meshheading:2246252-Sialic Acids,
pubmed-meshheading:2246252-Trisaccharides,
pubmed-meshheading:2246252-beta-Mannosidase
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pubmed:year |
1990
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pubmed:articleTitle |
Sialyl-alpha 2-6-mannosyl-beta 1-4-N-acetylglucosamine, a novel compound occurring in urine of patients with beta-mannosidosis.
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pubmed:affiliation |
University Children's Hospital Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.
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pubmed:publicationType |
Journal Article
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